Ribbing's disease is a rare form of sclerosing bone dysplasia characterized by exuberant yet benign endosteal bone, and periosteum formation in the diaphysis of long bones. Diagnosis relies on exclusionary criteria, as the primary clinical manifestations entail progressive pain unresponsive to analgesic therapy, accompanied by serological markers within normal ranges. Pain management constitutes the cornerstone of treatment, with surgery appearing to offer the most efficacious approach, despite the absence of a standardized therapeutic algorithm. The diagnostic and therapeutic delays associated with Ribbing's disease, reaching up to 16 years, exert a profound impact on patients' quality of life. Hence, the purpose of our work is to present a case report of Ribbing's disease and conduct a comprehensive literature review on the subject matter.
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Ribbing's disease is a rare form of sclerosing bone dysplasia characterized by exuberant yet benign endosteal bone, and periosteum formation in the diaphysis of long bones. Diagnosis relies on exclusionary criteria, as the primary clinical manifestations entail progressive pain unresponsive to analgesic therapy, accompanied by serological markers within normal ranges. Pain management constitutes the cornerstone of treatment, with surgery appearing to offer the most efficacious approach, despite the absence of a standardized therapeutic algorithm.
View Article and Find Full Text PDFThe effect of amlodipine and enalapril on blood pressure and neurohumoral activation in hypertensive patients with Ribbing's disease (multiple epiphysal dystrophy).
Clin Cardiol
February 2000
Rehaklinik Rheinfelden, Switzerland.
Background: In patients with Ribbing's disease (RD)--a form of multiple epiphysal dystrophy--hypertension is frequent, often severe, and accompanied by a relevant cardiac dysfunction.
Hypothesis: This study was undertaken to evaluate the contribution of the calcium antagonist amlodipine and of the angiotensin-converting-enzyme inhibitor enalapril to blood pressure regulation by studying their effect on neurohormonal activation.
Methods: Fifty hypertensive patients with RD were studied.
[Concurrence of familial ALS with Ribbing's disease].
Ryoikibetsu Shokogun Shirizu
October 1999
Fourth Department of Internal Medicine, Toho University School of Medicine.
[Clinical, humoral and scintigraphic assessment of a bisphosphonate as potential treatment of diaphyseal dysplasia: Ribbing and Cammurati-Engelmann diseases].
Medicina (B Aires)
June 1998
Sección Osteopatías Médicas, Hospital de Clínicas José de San Martín, Facultad de Medicina, Universidad de Buenos Aires, Argentina.
Cammurati-Engelmann's Disease or Progressive Diaphyseal Dysplasia (PDD), is a rare autosomal dominant disorder, sometimes non hereditable, which begins in childhood, and is characterized by symmetrical excess of osseous apposition in diaphysis and metaphysis of long bones. In severe cases skull and vertebrae could be involved. Clinically, patients refer limb pain, muscular weakness and atrophy, easy fatigability and waddling gait.
View Article and Find Full Text PDFCardiac involvement in Ribbing's disease.
Eur Heart J
August 1994
Division of Cardiology, Rehaklinik Rheinfelden, Switzerland.
Cardiac involvement was assessed in 14 patients with Ribbing's disease, a rare hereditary sclerosing bone dysplasia. When compared to age-, sex- and blood pressure-matched controls, the patients with Ribbing's disease had significant alterations in left ventricular systolic and diastolic function and an impaired exercise tolerance. Supraventricular and ventricular arrhythmias also tended to be more frequent in these patients than in controls.
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